Emergency Department Care

Avoid unnecessary use of antibiotics or antimotility agents during diarrheal illness. The use of these agents has been shown to increase the incidence of hemolytic uremic syndrome (HUS) because as motility slows, the gut is exposed to the toxins for a longer period of time. Additionally, antibiotic-induced injury to the bacterial membrane favors the acute release of large amounts of toxins. Use of antibiotics has been shown to increase the risk of full-blown HUS by 17-fold, and thus, the recommendation is to avoid its use, except in cases of sepsis.

Maintain fluid balance. In light of the diarrheal illness, fluid resuscitation is important, although one must avoid fluid overload. Watch for and treat hyperkalemia. If indicated, treat renal failure aggressively with hemodialysis.

Plasma exchange (plasmapheresis combined with fresh-frozen plasma replacement) is currently the treatment of choice. Plasma exchange is performed daily until remission is obtained. However, because 85% of children with hemolytic uremic syndrome recover after supportive therapy alone, plasma exchange is generally reserved for the most severe cases.

Eculizumab (Soliris) is the first treatment approved by the US Food and Drug Administration (FDA) (September, 2011) for adults and children with atypical hemolytic uremic syndrome (aHUS). Approval was based on data from adults and children who were resistant or intolerant to, or receiving, long-term plasma exchange/infusion. Data also included children (aged 2 mo to 17 y) who received eculizumab with or without prior plasma exchange/infusion. Eculizumab demonstrated significant improvement in platelet count from baseline (P = 0.0001). Thrombotic microangiopathy events were reduced, and maintained or improved kidney function was also reported.
[7, 8, 9]

In a study of 11 children (median age 22 months, range 11-175) with enterohemorrhagic E coli–positive HUS requiring dialysis, Pape and colleagues reported that early use of eculizumab appears to improve neurological outcome. All the study patients had seizures and/or were in a stupor or coma. Three patients died and of the surviving 8 patients, none experienced further seizures after the first dose of eculizumab. Three patients showed mild neurological impairment at discharge, while the remaining 5 showed no impairment.
[10]

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Consultations

Consult a hematologist and a nephrologist to help manage the case and an intensivist to admit the patient to an ICU setting, if necessary. In severe cases, consider consulting the renal transplant service if renal dysfunction persists.

Mark A Silverberg, MD, MMB, FACEP Assistant Professor, Associate Residency Director, Department of Emergency Medicine, State University of New York Downstate College of Medicine; Consulting Staff, Department of Emergency Medicine, Staten Island University Hospital, Kings County Hospital, University Hospital, State University of New York Downstate Medical Center